General Information About Pheochromocytoma and Paraganglioma
Table 1. Major Genetic Syndromes That Carry an Increased Risk of Pheochromocytoma continued...
Computed tomography (CT) imaging or magnetic resonance imaging (MRI) of the abdomen and pelvis (at least through the level of the aortic bifurcation) are the most commonly used methods for localization. Both have similar sensitivities (90%–100%) and specificities (70%–80%). CT imaging provides superior anatomic detail compared with MRI.
Additional functional imaging may be necessary if CT imaging or MRI fails to localize the tumor. It might also be useful in patients who are at risk for multifocal, malignant, or recurrent disease. 123 I-metaiodobenzylguanidine (MIBG) scintigraphy coupled with CT imaging provides anatomic and functional information with good sensitivity (80%–90%) and specificity (95%–100%).131 I-MIBG can be used in the same way, but the image quality is not as high as with 123 I-MIBG. Other functional imaging alternatives include 111 In-octreotide scintigraphy and 18 F-fluorodeoxyglucose positron emission tomography, both of which can be coupled with CT imaging for improved anatomic detail.
It is rare for localization of a catecholamine-secreting tumor to be unsuccessful if currently available imaging methods are used.
Prognosis and Survival
There are no clear data regarding the survival of patients with localized (apparently benign) disease or regional disease. Although patients with localized (apparently benign) disease should experience an overall survival approaching that of age-matched disease-free individuals, 6.5% to 16.5% of these patients will develop a recurrence, usually 5 to 15 years after initial surgery.[31,32,33]
Approximately 50% of patients with recurrent disease experience distant metastasis. The 5-year survival in the setting of metastatic disease (whether identified at the time of initial diagnosis or identified postoperatively as recurrent disease) is 40% to 45%.
Long-term follow-up is essential for all patients with pheochromocytoma or extra-adrenal paraganglioma, even when initial pathology demonstrates no findings that are concerning for malignancy.
- After resection of a solitary sporadic pheochromocytoma, patients should undergo baseline postoperative biochemical testing followed by annual lifelong biochemical testing.
- Patients who have undergone resection of a noncatecholamine-producing tumor should initially undergo annual imaging with computed tomography imaging or magnetic resonance imaging and periodic imaging with radiolabeled metaiodobenzylguanidine to monitor for recurrence or metastasis.
- Patients who have undergone resection of a pheochromocytoma or paraganglioma in the setting of a hereditary syndrome require lifelong annual biochemical screening in addition to routine screening for other component tumors of their specific syndrome.
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